Objective To explore the methods of evidence-based individualized treatment for a patient with oral pemphigus. Methods According to chnical problems, we searched The Coehrane Library （Issue 1,2005 ） , MEDLINE （1966 -Jan. 2005 ）, SUMSEAR.CH （Jan. 2005 ）, TRIPDATABASE （1997 - 2005 ）, CMCC （1994 - 2004 ）and handsearched four Chinease journals （starting pubhcation -2004）. Results No Systematic review was found. Six randomised controlled trials, two reviews of high quality and one guidehne were identified. We found that corticosteroid was the firsthne medicine in the treatment of pemphigus. However, corticosteroid at higher dose did not work more effectively than that at lower dose. Corticosteroid at middle-dose was preferred in treating oral pemphigus. Additionally, corticosteroid combined with immunosuppressants led to lower mortality. Among them, cyclophosphamide and azathioprine were recommended, and methopterin should be avoided. Plasma exchange in combination with corticosteroid at lowor middle-dose should not be recommended in conventional therapy. Chinese traditional treatment combined with Western medicine might work. A treatment with prednisone at the dose of 60 mg/d and in combination with azathioprine 100mg/d was made based on the evaluation of the data available and was proved optimal by 6 months following-up. Conclusions The therapeutic effect of pemphigus has been improved by evidence-based treatment.
ObjectiveTo learn the serum immunoglobulin G4 (IgG4) level in patients with pemphigus vulgaris (PV) in the active phase by detecting the IgG4 concentration in the patients with PV. MethodsWe collected the serum samples from patients with active-phase PV (including acute-onset and chronic active phases) between April and December 2014. The serum IgG4 level was detected by immuno-scatter turbidmetry, and the difference of serum IgG4 level between PV patients and normal subjects was assessed by Student's t-test. ResultsThirty-two patients with pemphigus vulgaris in the active phase were enrolled for this study, among whom 22 (including 8 males and 14 females) had normal serum IgG4 level, 8 (including 3 males and 5 females) higher and 2 (including 1 male and 1 female) lower. Compared with normal subjects, the average level of serum IgG4 in PV patients was not significantly different (P>0.05). The serum IgG4 level of acute-onset and chronic active PV patients was (1.015±0.825) g/L and (1.058±1.133) g/L, respectively, with no significant difference (P>0.05). ConclusionThe serum IgG4 level of patients with pemphigus vulgaris in the active phase can be normal, higher or lower. And there is no obvious difference in the serum IgG4 level between acute-onset and chronic active PV patients.
Objective To explore the clinical characteristics, diagnose and treatment of bronchiolitis obliterans (BO) associated with lymphoma and paraneoplastic pemphigus (PNP). Methods One patient with BO associated with lymphoma and paraneoplastic pemphigus was presented and literatures which searched through Wanfang Med online and PubMed on this subject were reviewed. Results A 44-year-old woman was admitted to this hospital because of oral ulcer for two years, and persistent dyspnea for fourteen months. She had been diagnosed with PNP one year earlier. Pulmonary function displayed severe obstructive ventilator dysfunction, negative response to the bronchodilator. Chest CT showed bronchiolectasis. The ratio of neutrophils increased in bronchoalveolar lavage fluid. The pathology of ultrasound-guided neck and inguinal lymph node biopsy showed non-Hodgkin’s lymphoma. Based on the clinical presentation, the image and pathology of this patient, the diagnosis of having PNP with BO secondary to the lymphoma was made. After 10 cycles of CHOP chemotherapy, lymphoma and PNP went into remission, but the patient died from respiratory failure. The literatures were reviewed and 15 patients were found, out of them 60% (9/15) were female, 47% (7/15) follicular lymphoma and 47% (7/15) diagnosis of clinical BO which did not be supported by pathology. Lymphoma-PNP-BO was a clinical syndrome and 67% of the initial presentations were PNP. Patients died within 7 to 60 months. Conclusions BO associated with lymphoma and PNP is rare and the most common initial presentation is PNP. BO is the major cause of death. Treatments included chemotherapy, corticosteroid and immunosuppressor, lymphoma and PNP can be alleviated, but BO is aggravating.